Secondary Polycythemia



The word polycythemia indicates increased red blood cells, white blood cells, and platelets. Most of the time, it is used in place of erythrocythemia, or pure red blood cell increase, such as in secondary polycythemia.

The term polycythemia is reserved for the myeloproliferative disorder called polycythemia vera, in which all 3 peripheral blood cell lines can be increased.[1, 2]

Erythrocytosis or erythrocythemia is a more specific term that is used to denote increased red blood cells.[3]


Increased hemoglobin and hematocrit values reflect the ratio of red blood cell mass to plasma volume. Any change in either the hemoglobin or the hematocrit can alter test results.

Relative polycythemia, or erythrocythemia, results from decreased plasma volume (G a isb ö ck syndrome). A true polycythemia or erythrocythemia results from increased red blood cell mass. Therefore, hemoglobin and hematocrit levels cannot accurately help make this distinction. Direct measurement of red blood cell mass is necessary to differentiate these conditions.

In primary polycythemia, the disorder results from a mutation expressed within the hematopoietic stem cell or progenitor cells, which drives the eventual accumulation of red blood cells. The secondary polycythemic disorders may be acquired or congenital; however, they are driven by circulating factors that are independent of the function of hematopoietic stem cells.



United States

The frequency of secondary polycythemia depends on the underlying disease.


The mortality and morbidity of secondary polycythemia depend on the underlying condition.




Secondary polycythemia is defined as an absolute increase in red blood cell mass that is caused by enhanced stimulation of red blood cell production. In contrast, polycythemia vera is characterized by bone marrow with an inherent increased proliferative activity.[1, 3, 5, 6, 7, 8, 9, 10] Approximately two thirds of patients with polycythemia vera have elevated white blood cell (granulocyte, not lymphocyte) counts and platelet counts.[11] No other causes of polycythemia/erythrocytosis are associated with elevated granulocyte or platelet counts.

Enhanced erythroid stimulation results from the following:

Laboratory Studies

Imaging Studies

Histologic Findings

Increased total red blood cell mass determines true polycythemia. Secondary causes must be identified individually.

Medical Care

The development of secondary erythrocytosis in response to tissue hypoxia is physiologic and probably beneficial to many patients. The expanded red blood cell mass may partially or totally compensate for the lack of oxygen delivery and result in tissue oxygenation to its normal level. However, limitation of compensatory increased red blood cells compromises circulation because of hyperviscosity when the hematocrit reaches levels higher than 60-65%. The latter leads to greater tissue hypoxia and EPO secretion, a continued increase in red blood cells, and further impairment of circulation.

Surgical Care

Some cases of secondary polycythemia are caused by conditions that can be ameliorated by surgical removal or correction.

Medication Summary

No medications are available to treat the blood disorder of secondary polycythemia. Treat the underlying health problem.




Srikanth Nagalla, MBBS, MS, Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

Disclosure: Nothing to disclose.


Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Disclosure: Nothing to disclose.

Specialty Editors

Karen Seiter, MD, Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Disclosure: Novartis Honoraria Speaking and teaching; Novartis Consulting fee Speaking and teaching; Ariad Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Disclosure: Nothing to disclose.


  1. Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. May 1 2004;69(9):2139-44. [View Abstract]
  2. Passamonti F. How to manage polycythemia vera. Leukemia. Dec 9 2011;[View Abstract]
  3. McMullin MF. The classification and diagnosis of erythrocytosis. Int J Lab Hematol. Dec 2008;30(6):447-59. [View Abstract]
  4. Esparcieux A, Francina A, Vital-Durand D. [Abnormal haemoglobins with high oxygen affinity in the differential diagnostics of polycythemia]. Rev Med Interne. Oct 2011;32(10):e105-7. [View Abstract]
  5. Balcerzak SP, Bromberg PA. Secondary polycythemia. Semin Hematol. Oct 1975;12(4):353-82. [View Abstract]
  6. Prchal JF, Prchal JT. Molecular basis for polycythemia. Curr Opin Hematol. Mar 1999;6(2):100-9. [View Abstract]
  7. Patnaik MM, Tefferi A. The complete evaluation of erythrocytosis: congenital and acquired. Leukemia. May 2009;23(5):834-44. [View Abstract]
  8. McMullin MF. The classification and diagnosis of erythrocytosis. Int J Lab Hematol. Dec 2008;30(6):447-59. [View Abstract]
  9. Zhan H, Spivak JL. The diagnosis and management of polycythemia vera, essential thrombocythemia, and primary myelofibrosis in the JAK2 V617F era. Clin Adv Hematol Oncol. May 2009;7(5):334-42. [View Abstract]
  10. Carillo S, Henry L, Lippert E, Girodon F, Guiraud I, Richard C, et al. Nested high-resolution melting curve analysis a highly sensitive, reliable, and simple method for detection of JAK2 exon 12 mutations--clinical relevance in the monitoring of polycythemia. J Mol Diagn. May 2011;13(3):263-70. [View Abstract]
  11. Squizzato A, Romualdi E, Middeldorp S. Antiplatelet drugs for polycythaemia vera and essential thrombocythaemia. Cochrane Database Syst Rev. Apr 16 2008;CD006503. [View Abstract]
  12. Lee FS. Genetic causes of erythrocytosis and the oxygen-sensing pathway. Blood Rev. Nov 2008;22(6):321-32. [View Abstract]
  13. Gonzales GF, Gasco M, Tapia V, Gonzales-Castañeda C. High serum testosterone levels are associated with excessive erythrocytosis of chronic mountain sickness in men. Am J Physiol Endocrinol Metab. Jun 2009;296(6):E1319-25. [View Abstract]
  14. Patakas DA, Christaki PI, Louridas GE, Sproule BJ. Control of breathing in patients with chronic obstructive lung diseases and secondary polycythemia after venesection. Respiration. 1986;49(4):257-62. [View Abstract]
  15. Calverley PM, Leggett RJ, McElderry L, Flenley DC. Cigarette smoking and secondary polycythemia in hypoxic cor pulmonale. Am Rev Respir Dis. May 1982;125(5):507-10. [View Abstract]
  16. Schwarcz TH, Hogan LA, Endean ED, et al. Thromboembolic complications of polycythemia: polycythemia vera versus smokers' polycythemia. J Vasc Surg. Mar 1993;17(3):518-22; discussion 522-3. [View Abstract]
  17. Percy MJ, Beard ME, Carter C, Thein SL. Erythrocytosis and the Chuvash von Hippel-Lindau mutation. Br J Haematol. Oct 2003;123(2):371-2. [View Abstract]
  18. Van Maerken T, Hunninck K, Callewaert L, et al. Familial and congenital polycythemias: a diagnostic approach. J Pediatr Hematol Oncol. Jul 2004;26(7):407-16. [View Abstract]
  19. Kralovics R, Indrak K, Stopka T, et al. Two new EPO receptor mutations: truncated EPO receptors are most frequently associated with primary familial and congenital polycythemias. Blood. Sep 1 1997;90(5):2057-61. [View Abstract]
  20. Ip F, di Pierro I, Brown R, et al. Trough serum testosterone predicts the development of polycythemia in hypogonadal men treated for up to 21 years with subcutaneous testosterone pellets. Eur J Endocrinol. Nov 10 2009;[View Abstract]
  21. Remacha AF, Montserrat I, Santamaria A, et al. Serum erythropoietin in the diagnosis of polycythemia vera. A follow-up study. Haematologica. Jul-Aug 1997;82(4):406-10. [View Abstract]
  22. Sondel PM, Tripp ME, Ganick DJ, Levy JM, Shahidi NT. Phlebotomy with iron therapy to correct the microcytic polycythemia of chronic hypoxia. Pediatrics. May 1981;67(5):667-70. [View Abstract]
  23. Kershenovich S, Modiano M, Ewy GA. Markedly decreased coronary blood flow in secondary polycythemia. Am Heart J. Feb 1992;123(2):521-3. [View Abstract]
  24. Lubarsky DA, Gallagher CJ, Berend JL. Secondary polycythemia does not increase the risk of perioperative hemorrhagic or thrombotic complications. J Clin Anesth. Mar-Apr 1991;3(2):99-103. [View Abstract]
  25. Menon D, York EL, Bornstein RA, Jones RL, Sproule BJ. Optimal hematocrit and blood viscosity in secondary polycythemia as determined from cerebral blood flow. Clin Invest Med. 1981;4(2):117-21. [View Abstract]
  26. Rapado I, Albizua E, Ayala R, Hernández JA, Garcia-Alonso L, Grande S, et al. Validity test study of JAK2 V617F and allele burden quantification in the diagnosis of myeloproliferative diseases. Ann Hematol. Sep 2008;87(9):741-9. [View Abstract]
  27. York EL, Jones RL, Menon D, Sproule BJ. Effects of secondary polycythemia on cerebral blood flow in chronic obstructive pulmonary disease. Am Rev Respir Dis. May 1980;121(5):813-8. [View Abstract]